Summary
The principal lesion in this case of chronic early infantile neuropathy is a modification of the Schwann cell resulting in the formation of a concentric network of lamellae formed by a double-layered sheet of basement membrane around numerous, large myelinated and unmyelinated fibers. The abnormalities in the myelin sheaths and the frequency of desmosomes are noteworthy. These peculiarities distinguish this case from other chronic neuropathies described up to now. Nevertheless, it is impossible to say at present whether the ultrastructural aspect of the nerve studied here is in any way specific.
Zusammenfassung
Die wesentlichen Veränderungen bei dem mitgeteilten Fall von chronischer frühinfantiler Neuropathie bestehen in einer Modifikation der Schwann-Zellen, die zur Bildung eines konzentrischen Netzwerkes von Lamellen führt. Dieses wird durch eine doppelkonturierte Hülle der Basalmembran um zahlreiche große bemarkte und unbemarkte Fasern gebildet. Die Anomalien der Markscheiden sowie die Häufigkeit von Desmosomen sind bemerkenswert. Diese Besonderheiten unterscheiden diesen Fall von anderen, bisher beschriebenen chronischen Neuropathien. Trotzdem erscheint es derzeit nicht möglich, eine Aussage über die Spezifität der ultrastrukturellen Befunde am untersuchten Nerven zu treffen.
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Lyon, G. Ultrastructural study of a nerve biopsy from a case of early infantile chronic neuropathy. Acta Neuropathol 13, 131–142 (1969). https://doi.org/10.1007/BF00687025
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DOI: https://doi.org/10.1007/BF00687025